Large Cell Neuroendocrine Cancer (LCNEC) of Uterine Cervix (original) (raw)

Large-cell neuroendocrine carcinoma of the uterine cervix--a clinicopathological study of five cases

PubMed, 2001

Objective: The present study describes 5 cases of large-cell neuroendocrine carcinoma (LCNEC) of the uterine cervix, evaluating their clinical features and pathological profiles. Methods: Clinical data were obtained from the patients' clinical files at the combined gynaecological-oncology unit of Johannesburg Hospital and the University of the Witwatersrand Medical School, Johannesburg, South Africa. A histopathological diagnosis was obtained after biopsy material from all 5 patients was examined microscopically and subjected to immunohistochemical staining with MNF116 (pankeratin) synaptophysin and chromagranin A, all of which are neuroendocrine markers. Two patients received pelvic radiotherapy only. None of the 5 patients in this series received chemotherapy or underwent surgery. Results: All 5 patients were adult females, with an average age of 57.3 years. The majority were multiparous, with the most common presenting complaint being vaginal bleeding. Three of the 5 patients presented with advanced-stage cervical carcinoma, with evidence of metastases in 2 of them. Treatment responses and long-term survival in our series proved to be disappointing as 3 of the 5 patients died in less than 6 months. On histopathological examination, all 5 tumours showed features of a high-grade poorly differentiated malignant neoplasm with ulceration and extensive tumour necrosis including trabecular and organoid growth patterns. All 5 neoplasms also showed strong immunoreactivity for MNF116, while their endocrine nature was confirmed by staining for synaptophysin in all cases. None of the tumours showed positive straining for chromagranin A. Conclusions: LCNECs are rare tumours and distinct from other neoplasms of the uterine cervix. The results of this study reaffirm the biologically aggressive nature of this uncommon tumour and its very unfavourable prognosis.

Gynecologic large cell neuroendocrine carcinoma: A review

Rare Tumors

Large cell neuroendocrine carcinomas (LCNEC) are rare, aggressive high-grade neuroendocrine neoplasms within the neuroendocrine cell lineage spectrum. This manuscript provides a detailed review of published literature on LCNEC of gynecological origin. We performed a PubMed search for material available on gynecologic LCNEC. We analyzed 104 unique cases of gynecologic LCNECs, of which 45 were cervical primary, 45 were ovarian, 13 were uterine, and 1 was vaginal. A total of 45 cases of cervical LCNEC were identified with a median age of 36 years. Median overall survival was 16 months. We identified 45 ovarian LCNEC cases in the published literature with a median age of 54 years. Median overall survival was 8 months. 13 LCNEC cases of uterine origin were identified; 12 out of 13 were of endometrial origin and the median age was 71 years. The majority of patients presented with Stage III/IV disease (stages I–IV were 31%, 8%, 38%, and 23%, respectively). Gynecologic LCNEC is an aggressiv...

Spectrum of neuroendocrine carcinomas of the uterine cervix, including histopathologic features, terminology, immunohistochemical profile, and clinical outcomes in a series of 50 cases from a single institution in India

Annals of Diagnostic Pathology, 2013

Neuroendocrine carcinoma cervix Small cell carcinoma cervix Large cell neuroendocrine carcinoma cervix Immunohistochemistry in cervix carcinomas Cervix cancer Neuroendocrine carcinomas of the cervix are uncommon, characterized by a histomorphological spectrum and, mostly, an aggressive clinical course. There are only few substantial studies on such cases documented from our country, where cervical cancer is the second most common cancer affecting women. Herein, we present a spectrum of 50 cervical neuroendocrine carcinomas, including histopathologic features, terminology, immunohistochemical (IHC) profile, and clinical outcomes, wherever available. Fifty tumors occurred in women, with their age ranging from 23 to 69 years (mean, 48.6 years; median, 46.5 years). Stagewise, among 25 cases, most cases (6, or 24%) presented with stage IB. Average tumor size was 4.7 cm. On histopathologic review, 26 tumors (52%) were classified as small cell carcinoma (SMCA); 14 (28%), as large cell neuroendocrine carcinomas (LCNECs); 4 (8%), as SMCA + LCNECs; and 6, as mixed carcinomas, including 3 tumors (6%) with SMCA and squamous cell carcinoma (SCC), 2 tumors (4%) with LCNEC and adenocarcinoma, and a single tumor (2%) with LCNEC and squamous cell carcinoma. On IHC performed in 41 tumors (82%), 36 tumors (87.8%) were positive for at least a single neuroendocrine marker, and 22 (53.6%) expressed 2 neuroendocrine markers. Synaptophysin was positive in 22 (59.4%) of 37 tumors; chromogranin, in 27 (72.9%) of 37; CD56, in 8 (100%) of 8; and neuron-specific enolase in 7 (87.5%) of 8 tumors. Treatment wise, among 30 patients (60%), 6 (20%) underwent surgery, including Wertheim hysterectomy (5) and simple hysterectomy (1); 8 (26.6%) underwent surgery with adjuvant treatment, and 10 patients (33.3%) were offered chemotherapy and/or radiotherapy. On follow-up (27 patients, or 54%) over 1 to 144 months, 16 patients (59.2%) were alive with disease over median duration of 9 months, and 7 (25.9%) were free of disease over median duration of 26.5 months. There were 5 recorded deaths. Thirteen tumors (48.1%) metastasized, most commonly to liver. In cases with early stage disease and adjuvant treatment, including radiotherapy, LCNEC histology fared well. This study forms the largest documented series on cervical neuroendocrine carcinomas from our country, testifying the current histopathologic classification system. Although SMCAs can be recognized on morphology, LCNECs need to be correctly identified because these can be misdiagnosed in the absence of neuroendocrine markers. Synaptophysin, chromogranin, and CD56 are optimal IHC markers. Small cell carcinomas, pure or mixed, are relatively more aggressive. All these tumors are best treated with multimodal therapy. Early stage disease treated with radical surgery and adjuvant treatment seems to increase survival. Despite aggressive treatment, prognosis is dismal.

High-Grade Neuroendocrine Carcinoma of the Cervix

Gynecologic and Obstetric Investigation, 2005

Neuroendocrine tumor of the uterine cervix is a rare and aggressive malignancy. Despite controversial, multimodal treatment methods, prognosis and treatment outcomes are worst in advanced stages. We report an early-stage case treated with the multimodal approach. The nomenclature proposed for this tumor type in 1997 by the College of American Pathologists still has some points of discussion. As in our case some of the tumors cannot be defined exactly into small or large cell types, and this causes confusion at least for nomenclature purposes. A ‘mixed’ type for this tumor may be appropriate.

Small Cell Carcinoma of the Uterine Cervix with Large Cell Neuroendocrine Carcinoma Component

Gynecologic Oncology, 1998

A case of cervical small cell carcinoma (SCC) with large cell neuroendocrine carcinoma (LCNC) differentiation is presented. A 35-year-old Taiwanese woman was diagnosed as having stage IIB bulky SCC confirmed by cervical biopsy and underwent induction combination chemoradiotherapy followed by hysterectomy. The pathology of the cervical tumor after the initial treatment showed the residual tumor to be LCNC instead of SCC. Histochemistry, immunohistochemistry, and electron microscopy demonstrated presence of neuroendocrine differentiation on both the biopsy and the surgical specimens. Following surgical resection a course of adjuvant chemotherapy and a local radiation boost were added. Despite complete local control, she developed brain metastasis 8 months later and vertebral spread soon thereafter. The pathology of the brain tumor showed pure SCC. The patient died 19 months after diagnosis and 13 months after completion of treatment. This case suggests that SCC with LCNC component has a similar clinical course as a pure SCC.

Neuroendocrine tumors of the uterine cervix

European Journal of Obstetrics & Gynecology and Reproductive Biology, 2000

Four main clinicopathologic features of neuroendocrine tumors (NETs) of the cervix may be stressed: primary diagnosis at an advanced stage, early nodal metastasis even for low disease, early failure of appropriate local treatment (surgery and / or radiation therapy) and aggressive clinical treatment. Five patients with NET of the uterine cervix (small cell carcinoma type) are reported (one stage I, two stages II, one stage III and one stage IV). One patient was treated by surgery combined with radiation therapy, one by surgery combined with chemotherapy and one by surgery with radiation therapy and chemotherapy. Two patients received radiation therapy alone. Three early stage patients are alive with no evidence of disease 8, 26 and 41 months after diagnosis. The two patients with advanced stage died of disease, 3 and 12 months respectively, after diagnosis. Combination chemotherapy (cisplatin and etoposide) is warranted in disseminated NETs. Neoadjuvant or adjuvant chemotherapy should be combined with radiation therapy and surgery even in early stages.

Large cell neuroendocrine carcinoma of cervix: A rare and distinct clinicopathological entity

International Journal of Medical Reviews and Case Reports, 2020

Neuroendocrine carcinoma of the cervix is rare, and prognosis is very poor. Because of its rarity, effective treatment of neuroendocrine carcinoma of the cervix has not been established. It has distinct patterns of metastasis, but can be successfully treated with radical surgery and platinum-based chemotherapy. We report a 50-year-old woman who was diagnosed with large cell neuroendocrine carcinoma of the cervix and underwent radical surgery followed by adjuvant chemoradiation therapy. She experienced recurrence several times and to different sites, with sequential metastasis to the lung, breast, and retroperitoneum, but nevertheless survived more than 5 years.

NEUROENDOCRINE CARCINOMA OF THE UTERUS

Neuroendocrine carcinoma of the cervix and endometrium are very rare and aggressive tumours. The present case study is of a 52 year old female who was a known case of hypertension and diabetes mellitus and presented to Sir Ganga Ram Hospital with postmenopausal bleeding per vaginum with moderately differentiated adenocarcinoma reported on endometrial biopsy done outside. Patient underwent total Laparoscopic Radical Hysterectomy with staging and bilateral pelvic lymphadenectomy. Her final FIGO staging was stage Ib. Histopathology confirmed poorly differentiated endometroid carcinoma of fundus of uterus involving up to 2/3 rd of myometrium with immunohistochemistry positive for cytokeratin, synaptophysin and 10-15% of tumour cells positive for Ki67, thus confirming the diagnosis of neuroendocrine carcinoma. So, it was concluded that the diagnosis of neuroendocrine carcinoma has to be kept in mind while treating patients with bleeding per vaginum especially in perimenopausal and postmenopausal group. A multispeciality approach is a key to the management on such a group of patients, to yield the best of results.